Hemolytic Anemias Quiz
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Questions and Answers

Which of the following conditions is classified under extrinsic abnormalities of hemolytic anemias?

  • Immuno-hemolytic Anemia (correct)
  • Thalassemia
  • Hereditary Spherocytosis
  • Sickle Cell Anemia

    • Sickle cell anemia is classified as an intrinsic abnormality of hemolytic anemia.

    False

    What is the primary issue in hereditary spherocytosis that affects red blood cell morphology?

    Membrane skeleton proteins

    In sickle cell anemia, red blood cells become __________ shaped during times of low oxygen.

    <p>sickle</p> Signup and view all the answers

    Match the following disorders with their characteristics:

    <p>Hereditary Spherocytosis = Defect in membrane skeleton proteins Sickle Cell Anemia = Abnormal globin synthesis Thalassemia = Deficient globin synthesis Immuno-hemolytic Anemia = Antibody mediated hemolysis</p> Signup and view all the answers

    What is one common clinical feature of hereditary spherocytosis?

    <p>Splenomegaly</p> Signup and view all the answers

    Sickle cell anemia is the least common familial hemolytic anemia in the world.

    <p>False</p> Signup and view all the answers

    What is the primary reason for increased osmotic fragility in RBCs in hereditary spherocytosis?

    <p>Loss of cell membrane relative to cell volume</p> Signup and view all the answers

    The gene frequency for sickle cell anemia can approach 30% in regions where malaria is endemic, due to its protective effect against ______.

    <p>Plasmodium falciparum malaria</p> Signup and view all the answers

    Match the following features or concepts related to sickle cell anemia:

    <p>HbS = Substitution of valine for glutamic acid Normal hemoglobin = Tetramer composed of two pairs of similar chains Epidemiology = Common in regions with malaria Morphology = Sickled RBCs under low oxygen tension</p> Signup and view all the answers

    What treatment option provides relief for symptomatic patients with hereditary spherocytosis?

    <p>Splenectomy</p> Signup and view all the answers

    Aplastic crises in hereditary spherocytosis are often due to infections like parvovirus B19.

    <p>True</p> Signup and view all the answers

    What percentage of normal adult red cells is composed of HbA?

    <p>96%</p> Signup and view all the answers

    Which of the following is a characteristic of hereditary spherocytosis?

    <p>Reduced membrane stability of RBCs</p> Signup and view all the answers

    Jaundice is a common clinical feature of hemolytic anemias.

    <p>True</p> Signup and view all the answers

    What type of inheritance pattern is commonly associated with hereditary spherocytosis?

    <p>autosomal dominant</p> Signup and view all the answers

    In hereditary spherocytosis, RBCs become less __________ and vulnerable to destruction by macrophages.

    <p>deformable</p> Signup and view all the answers

    Match the following characteristics with their associated condition:

    <p>Spherocytes = Hereditary Spherocytosis Turbulence by a defective valve = Intravascular Hemolysis Bilirubin-rich gallstones = Extravascular Hemolysis Hemoglobinemia = Intravascular Hemolysis</p> Signup and view all the answers

    What is the life span of red blood cells in individuals with sickle cell anemia?

    <p>20 days</p> Signup and view all the answers

    Which of the following tests is most likely to be increased in cases of hemolytic anemia?

    <p>LDH</p> Signup and view all the answers

    In homozygotes, HbS completely replaces HbA.

    <p>True</p> Signup and view all the answers

    Patients with hereditary spherocytosis will exhibit hemoglobinemia.

    <p>False</p> Signup and view all the answers

    What causes the distortion of red blood cells in sickle cell anemia?

    <p>Polymers formed by HbS molecules on deoxygenation</p> Signup and view all the answers

    What is the primary mechanism leading to the destruction of RBCs in hereditary spherocytosis?

    <p>Splenic sequestration</p> Signup and view all the answers

    In hereditary spherocytosis, the mutations mainly involve proteins such as __________, band 3, and spectrin.

    <p>ankyrin</p> Signup and view all the answers

    The sickling of red blood cells is initially ______ upon reoxygenation.

    <p>reversible</p> Signup and view all the answers

    What condition is characterized by jaundice, hyperbilirubinemia, and splenomegaly?

    <p>Hereditary Spherocytosis</p> Signup and view all the answers

    Match the following consequences of sickle cell anemia with their descriptions:

    <p>Chronic hemolytic anemia = Reduction in red blood cell lifespan Microvascular obstructions = Ischemic tissue damage due to stuck RBCs Acute chest syndrome = Pulmonary complications from vaso-occlusion Autosplenectomy = Loss of spleen function by adulthood</p> Signup and view all the answers

    Which factor enhances vaso-occlusion in sickle cell anemia?

    <p>Infection</p> Signup and view all the answers

    Individuals with sickle cell anemia can experience pain crises due to microvascular occlusions.

    <p>True</p> Signup and view all the answers

    What is the primary mutation responsible for sickle cell anemia?

    <p>Point mutation at position 6 causing valine substitution</p> Signup and view all the answers

    In sickle cell anemia, the release of _______ leads to the loss of potassium and water in red blood cells.

    <p>calcium</p> Signup and view all the answers

    What occurs to red blood cells over time in sickle cell anemia due to damage?

    <p>They become irreversibly sickled</p> Signup and view all the answers

    Study Notes

    Pathophysiology PHMU 534, Lecture 10: RBC Disorders I

    • Course title: Pathophysiology PHMU 534
    • Lecture: 10
    • Topic: Red Blood Cell (RBC) Disorders
    • Lecturer: Dr. Radwa Sabry

    Competencies

    • Domain 1: Fundamental Knowledge
      • Demonstrate understanding of pathophysiological mechanisms of diseases.
      • Use correct medical terminology in pharmacy practice.
      • Relate disease mechanisms to clinical manifestations and possible complications.
      • Utilize scientific literature and interpret information for professional decision-making.
    • Domain 2: Professional and Ethical Practice
      • Recognize physician roles in the healthcare team and perform responsibilities in compliance with professional structure.
    • Domain 3: Pharmaceutical Care
      • Apply body function principles and genomics to manage diseases.
      • Relate etiology, epidemiology, pathophysiology, diagnosis and clinical features to guide pharmacotherapeutic approaches.
    • Domain 4: Personal Practice
      • Demonstrate responsibility for team performance and provide peer evaluation, expressing time management skills.
      • Independently retrieve and critically analyze information, and work effectively within a team.
      • Demonstrate effective communication (verbal, non-verbal, and written) within the professional healthcare team, patients, and communities.
      • Utilize contemporary technologies and media to present effectively.
      • Engage in independent learning for continuous professional development.

    Outline

    • Overview of Anemia
      • Blood loss anemia (hemorrhage)
      • Hemolytic anemias
        • Hereditary spherocytosis
        • Sickle cell anemia
        • Thalassemia
        • Glucose-6-phosphate dehydrogenase deficiency
        • Immunohemolytic anemia
      • Anemia of diminished erythropoiesis
        • Iron deficiency anemia
        • Aplastic anemia

    Causes of Anemia

    • Increased RBC destruction (Hemolytic Anemia)
      • Intrinsic (Intracorpuscular)
      • Extrinsic (Extracorpuscular)
    • Decreased RBC production
      • Inadequate supply (Iron deficiency)
      • Disturbed proliferation and maturation of erythroblasts
      • Marrow replacement and infiltration
    • Bleeding
      • Chronic (e.g., GI lesions, gynecological disturbances)

    RBC Reference Ranges

    • Hemoglobin (Hb): (g/dL) Men: 13.2-16.7, Women: 11.9-15.0
    • Hematocrit (Hct): (%) Men: 38-48, Women: 35-44
    • Red blood cell count: (x106/μL) Men: 4.2-5.6, Women: 3.8-5.0
    • Other relevant values(MCV, MCH, MCHC, RDW) are also included.

    Morphology

    • Normal erythrocyte: Size 6-9µm, MCV 80-97fL
    • Microcytic hypochromic: Size <6µm, MCV <80fL
    • Normocytic normochromic: Size 6-9µm, MCV 80-97fL
    • Macrocytic: Size >9µm, MCV >100fL

    Anemia of Blood Loss

    • Acute blood loss
      • If >20% blood volume lost, immediate threat is hypovolemic shock, not anemia
      • RBCs are normocytic and normochromic
      • Erythropoietin level rises
    • Chronic blood loss
      • Iron stores gradually depleted
      • Iron deficiency anemia
      • Microcytic and hypochromic anemia

    Hemolytic Anemias

    • Intrinsic abnormalities (intracorpuscular)
      • Membrane skeleton proteins (e.g., hereditary spherocytosis)
      • Enzyme deficiency (e.g., G6PD deficiency)
    • Extrinsic abnormalities (extracorpuscular)
      • Abnormal globin synthesis (e.g., sickle cell anemia, thalassemias)
      • Antibody mediated (e.g., immune hemolytic anemia)

    Features of Hemolytic Anemias

    • Decreased RBC lifespan, leading to premature destruction
    • Erythroid hyperplasia in the bone marrow (compensation)
    • Reticulocytosis
    • Extramedullary hematopoiesis in severe cases (liver, spleen, lymph nodes)

    RBC Hemolysis

    • Intravascular hemolysis
      • Mechanical forces (e.g., defective heart valve)
      • Biochemical (e.g., exposure to toxins)
      • Jaundice, hyperbilirubinemia (unconjugated), haptoglobin decrease, hemosiderinuria, hemoglobinemia, hemoglobinuria, LDH increase.
    • Extravascular hemolysis
      • Reduction in RBC deformability
      • Jaundice, hyperbilirubinemia (unconjugated), haptoglobin decrease (if severe), bilirubin-rich gallstones, no hemoglobinemia, no hemoglobinuria, splenomegaly

    Hereditary Spherocytosis

    • Pathogenesis: Mutations in membrane proteins (ankyrin, band 3, spectrin) reduce membrane stability
    • Morphology: Spherocytes (lack central pallor)
    • Clinical features: Moderate anemia, jaundice, splenomegaly, aplastic crises (parvovirus B19), increased osmotic fragility.
    • Treatment: Splenectomy (balancing the risk of infection). Partial splenectomy is increasingly common, as it can improve outcomes, while protecting against sepsis.

    Sickle Cell Anemia

    • Pathogenesis: Point mutation in β-globin gene (Glu → Val) leads to abnormal hemoglobin (HbS), polymer formation and sickling under deoxygenation
    • Consequences: Hemolysis, vaso-occlusion, pain crises, organ damage. Microvascular obstructions
    • Morphology: Sickled cells (elongated crescentic or sickle-shaped)
    • Clinical features: Chronic hemolytic anemia, episodic pain crises, acute chest syndrome, stroke, splenic sequestration, (autosplenectomy likely by adulthood), aplastic crises (parvovirus B19), infections,
    • Factors affecting sickling: intracellular concentration of HbS, presence of other hemoglobins (HbF, HbA2) and co-existence of α-thalassemia which reduces sickling.
    • Epidemiology: Common in malaria-endemic regions due to protective effect; also present in other populations. High frequency in specific geographic areas.

    Sickle cell anemia factors affecting sickling:

    • The presence of other hemoglobin (HbF, HbA2)
    • Intracellular concentration of HbS
    • The transit time for RBCs through the microvasculature (slow blood flow)

    Other Topics (from the slides)

    • Epidemiology, normal hemoglobin, references are also included in detailed notes

    • Clinical features of both conditions are covered.

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    Lecture 10 RBCs Diseases PDF

    Description

    Test your knowledge on extrinsic and intrinsic abnormalities of hemolytic anemias. Explore the characteristics, clinical features, and treatments related to disorders like sickle cell anemia and hereditary spherocytosis. This quiz will challenge your understanding of red blood cell morphology and the genetic factors involved.

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